Subacute compressive myelopathy secondary to extramedullary hematopoiesis

Mielopatia subaguda compressiva secundária a hematopoiese extramedular

A 25-year-old man presented with subacute onset paraparesis with sphincter disturbance. His past medical history unvealed β-thalassemia intermedia (compound heterozygote IVS 1-5) and hereditary persistence of fetal hemoglobin type 2. His neurological examination disclosed a complete spinal cord syndrome without clear thoracic sensory level. Spinal cord imaging showed severe thoracic spinal stenosis secondary to paraspinal extramedullary hematopoiesis (Figure). Spinal cord compression is a leading cause of myelopathy in patients with multiple progressive paravertebral masses in chronic extramedullary hematopoiesis¹, described in hereditary hemoglobinopathies, lymphoproliferative disorders, myelofibrosis and polycythemia vera².

Figure
Non-contrast-enhanced sagittal MRI of the spine at thoracic level disclosing multiple solid masses in the paravertebral spine with intermediate signal on T2-weighted (A; asterisks) and on T1-weighted images (B; arrows). Contrast-enhanced T1-weighted sagittal MRI of the spine showing enhancement of the solid masses described in A and B (C; filled arrowhead). Axial T2-weighted MRI of the spine evinced severe thoracic spinal stenosis and multiple paravertebral masses (D; non-filled arrowhead).

References

• ¹ Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia. Eur Spine J 2010;19:871-878.
• ² Haidar R, Mhaidli H, Musallam KM, Taher AT. The spine in beta-thalassemia syndromes. Spine 2012;37:334-339.

Publication Dates

• Publication in this collection
  Sept 2014

History

• Received
  12 Apr 2014
• Reviewed
  01 May 2014
• Accepted
  16 June 2014